Audiological performance after cochlear implantation in children with inner ear malformations.

Objective: To prove that cochlear implantation is a beneficial method of rehabilitation in deaf children with malformations of the inner ear. Design: The evaluation of auditory responses to speech (EARS) test battery was performed on the children in this study after an average implant use of 3 years. Results: Individual results of six children with inner ear anomalies receiving cochlear implants are presented in this study. Three of the patients showed an incomplete partition (Mondini dysplasia), one had a cochlear hypoplasia and two suffered from an intraoperative cerebrospinal fluid leak. The majority of the children in this study are successful implant users. Wherever possible, test scores are included and subjective case reports given. Conclusion: Results are similar to those in children with normal cochleas, therefore inner ear malformations found in as many as 20% of patients with congenital sensorineural hearing loss are no contraindication for cochlear implantation. Nevertheless, factors influencing the success of implantation are multiple, including a thorough preoperative radiological examination, a well-performed surgery and an individually tailored postoperative rehabilitation programme.